Quantitative Assessment of Serum Copper Status in Children with Thalassemia Major

Objective: Support of ordinary degrees of copper, has a significant influence in reducing the morbidities related with thalassemia major. Levels of this component has been seen as modified in this incessant transfusion subordinate infection from everywhere throughout the world, however with broadly factor results. Other than rehashed blood transfusions and utilization of chelating operators, the level of copper in thalassemia patients are influenced by hereditary and dietary factors likewise, convincing us to embrace this investigation in our population. The Objectives of this study was to appraise of serum levels of copper in children with beta-thalassemia major. Methods: In this observational comparative research design, all children between 5 to 15 years affected by beta thalassemia major (30 patients) and 30 normal children were evaluated for serum copper levels in the Department of Pediatrics, DMCH and attended at Thalassemia Center, DSH (Dhaka Shishu Hospital). Serum measurements for copper were performed using Atomic Absorption Spectrophotometer (Varian, AA 240 FS, USA) in Analytical Chemistry Laboratory, Chemistry Division, Atomic Energy Centre, Dhaka, Bangladesh. Results: The minimum, maximum, and the mean concentration of serum copper in patients were 103.50, 239.94, and 147.55 ± 35.17 µg/dl and in control group, those were 58.75, 192.43, and 123.85± 33.19 µg/dl respectively. There was no significant difference in serum copper level in the β thalassemia group and the control group (p=0.54) (P=0.3). Conclusion: This investigation revealed that there is no copper inadequacy. Further assessment in such manner is suggested.

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Relationship between Vitamin B9 (Folic Acid), Vitamin B12 (Cobalamin), and Peripheral Neuropathy in Children with Beta-Thalassemia Major

Global Medical & Health Communication (GMHC) ◽

10.29313/gmhc.v9i2.8106 ◽

2021 ◽

Vol 9 (2) ◽

Author(s):

Uni Gamayani ◽

Titin Junaidi ◽

Nushrotul Lailiyya ◽

Nur Suryawan ◽

Nanan Sekarwana

Keyword(s):

Folic Acid ◽

Peripheral Neuropathy ◽

Vitamin B12 ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Control Group ◽

Case Group ◽

Beta Thalassemia Major ◽

Vitamin B9 ◽

Significant Difference

Vitamin B9 (folic acid) and B12 (cobalamin) are essential vitamins that play roles in the process of hematopoiesis and maintaining the function of peripheral nerves. Therefore, these deficiencies may create a risk for peripheral neuropathy in beta-thalassemia major patients. The purpose of this study is to determine the relationship between vitamin B9 level, vitamin B12 level, and peripheral neuropathy in beta-thalassemia major children. It was an observational analytical study with a case-control design has been conducted at Dr. Hasan Sadikin General Hospital Bandung, Indonesia, in May–July 2019. There were 47 beta-thalassemia major children with peripheral neuropathy (case) and 41 healthy children (control). All subjects completed a general demographic questionnaire, underwent neurological examination, and were tested for vitamin B9 and B12 serum levels. Data were then analyzed using the unpaired t test to compare the vitamin levels between both groups and Spearman’s rank correlation test to investigate the correlation between vitamin levels and the number of affected nerves in the case group. Comparison of folic acid levels in the case group (21.52±6.22 ng/mL) and the control group (23.81±7.51 ng/mL) showed no significant difference (p=0.19). In contrast, cobalamin in the case group (288.57±168.61 ng/mL) and the control group (385.95±197.48 ng/mL) showed a significant difference (p=0.01). In addition, there was a moderate correlation (p=0.004, r=0.41) between folic acid level and the number of motoric nerves affected in the case group. In conclusion, cobalamin level correlates with peripheral neuropathy in beta-thalassemia major patients, and folic acid level correlates with the number of affected nerves, especially motoric nerves. HUBUNGAN ANTARA VITAMIN B9 (ASAM FOLAT), VITAMIN B12 (KOBALAMIN), DAN NEUROPATI PERIFER PADA ANAK DENGAN TALASEMIA BETA MAYORVitamin B9 (asam folat) dan B12 (kobalamin) merupakan vitamin esensial yang berperan dalam proses hematopoiesis dan menjaga fungsi saraf tepi. Defisiensi vitamin ini dapat menimbulkan risiko neuropati perifer pada pasien talasemia beta mayor. Tujuan penelitian ini mengetahui hubungan antara kadar vitamin B9, vitamin B12, dan neuropati perifer pada anak talasemia beta mayor. Metode penelitian ini adalah analitik observasional dengan rancangan studi kasus kontrol yang dilakukan di RSUP Dr. Hasan Sadikin Bandung, Indonesia pada Mei–Juli 2019. Terdapat 47 anak talasemia beta mayor dengan neuropati perifer (kelompok kasus) dan 41 anak sehat (kelompok kontrol). Seluruh subjek penelitian mengisi kuesioner demografi umum, menjalani pemeriksaan fisis neurologis, serta dilakukan tes kadar vitamin B9 dan B12 serum. Uji t test tidak berpasangan digunakan untuk membandingkan kadar vitamin pada kedua kelompok dan uji korelasi Spearman untuk membandingkan kadar kedua vitamin tersebut dengan jumlah saraf yang terkena pada kelompok kasus. Perbandingan kadar asam folat kelompok kasus (21,52±6,22 ng/mL) dan kelompok kontrol (23,81±7,51 ng/mL) menunjukkan perbedaan yang tidak bermakna (p=0,19), sedangkan perbandingan kadar kobalamin kelompok kasus (288,57±168,61 ng/mL) dan kelompok kontrol (385,95±197,48 ng/mL) menunjukkan perbedaan yang bermakna (p=0,01). Selain itu, terdapat korelasi sedang (p=0,004; r=0,41) antara kadar asam folat dam jumlah saraf motorik yang terkena pada kelompok kasus. Kesimpulan, kadar kobalamin berhubungan dengan neuropati perifer pada penderita talasemia beta mayor dan kadar asam folat berhubungan dengan jumlah saraf yang terkena, terutama saraf motorik.

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Adaptive Functioning and Psychosocial Problems in Children with Beta Thalassemia Major

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2018.367 ◽

2018 ◽

Vol 6 (12) ◽

pp. 2337-2341 ◽

Cited By ~ 2

Author(s):

Fatma A. Alzaree ◽

Manal A. Shehata ◽

Maged A. El Wakeel ◽

Inas R. El-Alameey ◽

Mones M. AbuShady ◽

...

Keyword(s):

Psychosocial Functioning ◽

Adaptive Functioning ◽

Thalassemia Major ◽

Adaptive Behaviour ◽

Beta Thalassemia ◽

Psychosocial Burden ◽

Normal Children ◽

Beta Thalassemia Major ◽

Significant Difference ◽

Behaviour Score

BACKGROUND: Beta thalassemia major is considered one of the serious health problems and the commonest hemoglobinopathy in Egypt that creates a burden not only on health system but also on the affected families and children who become vulnerable to emotional, social, psychological and behavioural problems. AIM: This study was designed to assess the psychosocial burden and the adaptive functioning in children with beta-thalassemia major. SUBJECTS AND METHODS: A group of 50 children with thalassemia major and 50 normal children matched for age and sex were included in a case-control study. Vineland Adaptive Functioning Scale was used to assess the adaptive functions; while the Pediatric Symptom Checklist (PSCL) was used to assess psychosocial morbidity. RESULTS: A group of 50 children aged 5-17 years old with thalassemia major, their mean age was 11.05 ± 3.8, showed a statistically significant lower total adaptive behaviour score and communication subscale score. All the mean values of adaptive behaviour for cases and controls were within the average values. Results from the PSCL revealed no significant difference between mean scores of children with thalassemia and controls. A score of attention domain was markedly higher in children with thalassemia. Internalising behaviour was the most dominant as it was detected in 10% of the patient group. CONCLUSION: Thalassemic patients had a relatively mild affection for adaptive and psychosocial functioning that can be explained by social and medical support they receive, which may increase their competence and psychological wellbeing.

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Comparison of Blood Pressure in Beta Thalassemia Major Patients with that of Control

International Journal of Current Research in Physiology and Pharmacology (IJCRPP) ◽

10.31878/ijcrpp.2020.42.01 ◽

2020 ◽

pp. 1-3

Author(s):

Sneha Mahadev Bhatkar ◽

Surendra S Shivalkar ◽

Sachin H Mulkutkar

Keyword(s):

Blood Pressure ◽

Systolic Blood Pressure ◽

Diastolic Blood Pressure ◽

Normal Subjects ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Control Group ◽

Study Group ◽

Beta Thalassemia Major ◽

Significant Difference

Introduction and Objectives : β-thalassemia major patients need to have repeated blood transfusions throughout their life for survival, which leads to so many complications. Heart disease is the most important complication and the main determinant of survival in these patients. Our study shows comparison of blood pressure between these patients and that of normal subjects. Material and Methods : Present study was a cross sectional type of study and consisted of 100 normal subjects (control group) and 100 patients of beta thalassemia major (study group). Blood pressure was recorded in both the groups using mercury sphygmomanometer and statistical analysis of the observations was done using Z test. Results : There was no statistically significant difference in the Systolic blood pressure (SBP) of study group as compared to control group whereas there was a difference in the Diastolic blood pressure (DBP) of study group and control group which was statistically significant (p value <0.0001). Conclusion : There was an overall reduction in the blood pressure of study group as compared to control group but the difference in SBP was statistically not significant whereas that of DBP was statistically significant. Key Words: Beta thalassemia major, Diastolic blood pressure, Systolic blood pressure

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Assessment of Subclinical Renal Glomerular and Tubular Dysfunction in Children with Beta Thalassemia Major

Children ◽

10.3390/children8020100 ◽

2021 ◽

Vol 8 (2) ◽

pp. 100

Author(s):

Asmaa A. Mahmoud ◽

Doaa M. Elian ◽

Nahla MS. Abd El Hady ◽

Heba M. Abdallah ◽

Shimaa Abdelsattar ◽

...

Keyword(s):

Cystatin C ◽

Kidney Injury ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Good Survival ◽

Control Group ◽

Healthy Children ◽

Serum Cystatin C ◽

Serum Cystatin ◽

Beta Thalassemia Major

Background: A good survival rate among patients with beta thalassemia major (beta-TM) has led to the appearance of an unrecognized renal disease. Therefore, we aimed to assess the role of serum cystatin-C as a promising marker for the detection of renal glomerular dysfunction and N-acetyl beta-D-glucosaminidase (NAG) and kidney injury molecule 1 (KIM-1) as potential markers for the detection of renal tubular injury in beta-TM children. Methods: This case-control study was implemented on 100 beta-TM children receiving regular blood transfusions and undergoing iron chelation therapy and 100 healthy children as a control group. Detailed histories of complete physical and clinical examinations were recorded. All subjected children underwent blood and urinary investigations. Results: There was a significant increase in serum cystatin-C (p < 0.001) and a significant decrease in eGFR in patients with beta-TM compared with controls (p = 0.01). There was a significant increase in urinary NAG, KIM-1, UNAG/Cr, and UKIM-1/Cr (p < 0.001) among thalassemic children, with a significant positive correlation between serum cystatin-C, NAG and KIM-1 as regards serum ferritin, creatinine, and urea among thalassemic patients. A negative correlation between serum cystatin-C and urinary markers with eGFR was noted. Conclusion: Serum cystatin-C is a good marker for detection of glomerular dysfunction. NAG and KIM-1 may have a predictive role in the detection of kidney injury in beta-TM children.

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Does copper deficiency predispose children to otitis media with effusion?

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20183691 ◽

2018 ◽

Vol 4 (5) ◽

pp. 1147

Author(s):

Maryam Amizadeh ◽

Sareh Molaei Moghbeli ◽

Hamed Reihani-Kermani ◽

Amir Reihani

Keyword(s):

Otitis Media ◽

Otitis Media With Effusion ◽

Graphite Furnace ◽

Serum Levels ◽

Serum Copper ◽

Copper Level ◽

Control Group ◽

Serum Selenium ◽

Cross Sectional ◽

Significant Difference

Background: Both otitis media with effusion (OME) and copper or selenium have been associated to the immune system, hence it could be proposed that cooper and/or selenium deficiency predisposes children to OME through immune cells dysfunction. The aim of this study was to determine the serum levels of Copper and Selenium in an immundeficient-linked disease - OME – in childhood.Methods: This cross-sectional study was conducted between July 2015 and December 2016 including 80 patients age 1 to 8 years old who were divided into two groups: otitis media with effusion (OME) consisted of 40 patients and the control (n=40). Patients who did not recover after three months conservative treatments were included. Serum levels of Copper and Selenium were measured using graphite furnace atomic absorption spectrometry. Results: Statistical analysis showed no significant differences regarding age and sex were noted between groups. Patients with OME had significantly lower serum copper levels than the control group (p=0.007). However, there was no statistically significant difference in serum selenium levels between groups. The analysis also showed a significant positive correlation between serum copper level and age (correlation coefficient=0.348, p=0.028), whereas, there was no correlation between the measured selenium level and age.Conclusions: The results of the present study showed that serum level of copper might play a role on development of otitis media with effusion in childhood.

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ALTERATION IN SERUM ZINC AND COPPER CONCENTRATIONS AND EFFECT OF ORAL THERAPEUTIC SUPPLEMENTATION OF ZINC ON TRANSFUSION DEPENDANT BETA THALASSEMIA MAJOR PATIENTS

International Journal of Clinical and Biomedical Research ◽

10.31878/ijcbr.2020.62.03 ◽

2020 ◽

pp. 10-12

Author(s):

Ghone Rahul A ◽

Ghodake S S ◽

Bhagart Sonali S ◽

Karnik A C

Keyword(s):

Copper Concentration ◽

Zinc Supplementation ◽

Serum Zinc ◽

Thalassemia Major ◽

Normal Serum ◽

Beta Thalassemia ◽

Copper Level ◽

Beta Thalassemia Major ◽

The Impact ◽

The Way

Zinc is one of the essential micronutrients in human and act as a cofactor for more than 300 enzymes and plays an essential role in human growth and development. It has been observed that there was low serum zinc and elevated copper level in β-thalassemia major compared with normal. Zinc deficiency is considered one of the main factors contributing to growth, cardiovascular diseases, and puberty disorders in β-thalassemic patients. Aim: The goal of the study was to scrutinize the impact of serum zinc and copper concentration in patients with beta-thalassemia major and also to observe the effect of zinc supplementation on transfusion dependent beta-thalassemia patients for six months. Method: 52 beta-thalassemia major patients were studied before and after supplementation of zinc for six months, and status was compared with 52 age and sex-matched healthy normal. Serum zinc and copper concentration were measured by atomic absorption spectrophotometry (AAS) method. Result: There was a significant depleted activity of serum zinc level (p<0.001), and the copper level was increased significantly (p<0.001) in patients when compared with normal. After six months of supplementation of zinc, there was a significantly enhanced zinc concentration (p<0.001),and copper was marginally increased (p>0.05) when compared with normal and baselines. Conclusion: Beta Thalassemia major children are on numerous blood transfusions all the way through their life. Due to this thalassemic children are at risk of secondary iron burden. This further leads to the enhanced oxidative stress. One of the way to may overcome this situation to supply regular zinc supplementation along with treatment, which may be helpful to manage the situation.

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The effect of bone marrow-derived mesenchymal stem cell co-transplantation with hematopoietic stem cells on liver fibrosis alleviation and survival in patients with class III β-thalassemia major

Stem Cell Research & Therapy ◽

10.1186/s13287-021-02242-8 ◽

2021 ◽

Vol 12 (1) ◽

Author(s):

Tahereh Rostami ◽

Amir Kasaeian ◽

Nasrollah Maleki ◽

Mohsen Nikbakht ◽

Azadeh Kiumarsi ◽

...

Keyword(s):

Stem Cells ◽

Liver Fibrosis ◽

Chronic Gvhd ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Class Iii ◽

Mixed Effect ◽

Hematopoietic Stem ◽

Beta Thalassemia Major ◽

Significant Difference

Abstract Background Hepatic fibrosis is a common complication in transfusion-dependent thalassemia patients. Data on the co-transplantation of mesenchymal stem cells (MSCs) with hematopoietic stem cells (HSCs) in beta-thalassemia major patients are scarce. Therefore, we aimed to evaluate the effect of co-transplantation of bone marrow-derived MSC with HSCs on the liver fibrosis alleviation and transplant outcomes in class III beta-thalassemia major. Methods Between April 1998 and January 2017, a total of 224 consecutive patients with class III beta-thalassemia major underwent allogeneic HSCT in the Research Institute for Oncology, Hematology and Cell Therapy, Tehran University of Medical Sciences, Tehran, Iran. To assess liver fibrotic changes after transplantation, 47 patients participated in the MSC plus HSC group and 30 patients in the HSC only group at the end of the follow-up period. All patients underwent laboratory tests, especially serum ferritin and liver function testing, hepatic T2* MRI, liver biopsy, and FibroScan before and 2 years after transplantation. Kaplan-Meier curves were derived to determine survival and were compared using the log-rank test. Repeated-measure, mixed-effect linear regression models were used to examine the changes in liver fibrosis over time. Results The 10-year OS rate was 71.84% in the mesenchymal group and 61.89% in the non-mesenchymal group (P value = 0.294), while the 10-year TFS rate was 63.64% in the mesenchymal group and 52.78% in the non-mesenchymal group (P value = 0.285). No significant difference was observed in the 10-year NRM, rejection rate, ANC engraftment, platelet engraftment, acute GvHD, and chronic GvHD between the two groups. In addition, the results of repeated-measure, mixed-effect linear regression models showed that none of the variables determining hepatic fibrosis had a significant difference between patients receiving MSCs and patients who did not receive MSCs. Conclusions Based on the results of this study, a single infusion of MSCs at the time of HSCT to patients with class III beta-thalassemia major could not significantly improve the liver fibrosis alleviation and transplantation outcomes, including OS, TFS, TRM, rejection rate, ANC engraftment, platelet engraftment, acute GvHD, and chronic GvHD.

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Dental Arch Dimensions in Subjects with Beta-thalassemia Major

The Journal of Contemporary Dental Practice ◽

10.5005/jp-journals-10024-1071 ◽

2011 ◽

Vol 12 (6) ◽

pp. 429-433 ◽

Cited By ~ 2

Author(s):

Othman M Yassin

Keyword(s):

Thalassemia Major ◽

Severe Form ◽

Beta Thalassemia ◽

Control Group ◽

Dental Arch ◽

Mean Values ◽

Mandibular Arch ◽

Beta Thalassemia Major ◽

Males And Females ◽

And Control

ABSTRACT Aim Thalassemia is a group of inherited hemoglobinopathies with thalassemia major representing the severe form of the disease characterized by craniofacial deformities. The aim of this study is to provide a detailed description of dental arch dimensions in subjects with thalassemia major. Subjects and methods The sample consisted of 43 thalassemic subjects, 24 males and19 females, aged 7.3 to 15.4 years (mean ± SD = 10.6 ± 3.5 years) and control group matched by age and sex. Dental casts of the participants were measured for arch lengths, arch widths, using a digital sliding caliper. Student t-test was used for comparison of mean values between males and females as well as between thalassemic and control groups. Results The results show that all means of maxillary and mandibular arch dimensions in thalassemic males and females were smaller than their controls, with 14 of the 16 comparisons being statistically significant (ranged from p < 0.05 to p < 0.001). The segmental arch lengths in the maxilla and mandible of thalassemic group were reduced by an average of 2.59 and 2.55 mm respectively, compared with the control group. The mean maxillary and mandibular arch depths (lengths) in the thalassemic group were shorter by 3.21 and 2.63 mm respectively, relative to the controls (p < 0.001). All arch widths thalassemic patients were significantly reduced by an average ranged from 1.33 to 1.90 mm in the maxilla and 1.37 to 1.77 mm in the mandible. Conclusion The present study showed that the maxillary and mandibular dental arches dimensions are significantly reduced in patients with thalassemia major compared with healthy control subjects. Clinical significance Changes in the size of dental arches and tooth dimensions in thalassemic patients have an impact on the occlusal relationships. These changes should be taken into account when planning orthodontic treatment and orthognathic surgery. How to cite this article Hattab FN, Yassin OM. Dental Arch Dimensions in Subjects with Beta-thalassemia Major. J Contemp Dent Pract 2011;12(6):429-433.

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Evaluation of Erythroferrone, Hepcidin, and Iron Overload Status in Iraqi Transfusion-dependent β-Thalassemia Major Patients

10.20944/preprints201912.0356.v1 ◽

2019 ◽

Author(s):

Hasan Smesam ◽

Hasan Qazmooz ◽

Sareh Arjmand ◽

Hussein Kadhem Al-Hakeim ◽

Seyed Omid Ranaei‐Siadat,

Keyword(s):

Iron Overload ◽

Iron Status ◽

Iron Chelation ◽

Fold Increase ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Control Group ◽

Beta Globin ◽

Beta Thalassemia Major ◽

Globin Chains

Beta thalassemia major (β-TM) disorder characterized by the lack, or severe reduction in the production of hemoglobin β-globin chains. The standard protocol for the management of β-TM is blood transfusion and iron chelation therapy to reduce the iron overload state. The present study aimed to investigate the relationships between two iron regulatory hormones, hepcidin (HEPC) and erythroferrone (ERFE) levels and iron status parameters (ISPs) in Iraqi patients with β-TM. ISPs and hormones were measured in sixty patients and compared with thirty healthy controls. The results indicated significant changes in different iron status parameters, while ferritin (FRT) with the ~11 fold increase showed the most change. Significant reduction in HEPC and increase in ERFE levels were detected in patients as compared to the control group, while no direct correlation was identified with the other measured ISPs. Receiver operating characteristic (ROC) analysis showed that the z-score of the composite of ERFE+FRT has a full diagnostic ability for β-TM. In conclusion, our finding indicated the correlation between different ISPs, FRT as the leading predictor of iron overload and tow main iron regulatory hormones.

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Investigation of Pancreatic Sonography Findings in Patients With Beta-Thalassemia Major

Journal of Diagnostic Medical Sonography ◽

10.1177/8756479321989662 ◽

2021 ◽

pp. 875647932198966

Author(s):

Razieh Behzadmehr ◽

Iraj Shahramian ◽

Pouya Ostad Rahimi ◽

Mahboobeh Sheikh ◽

Soosan keikha ◽

...

Keyword(s):

Statistical Tests ◽

Pancreatic Head ◽

Thalassemia Major ◽

Cross Sectional Study ◽

Beta Thalassemia ◽

Control Group ◽

P Value ◽

Case Group ◽

Cross Sectional ◽

Beta Thalassemia Major

Objective: Some structural abnormalities have been reported on sonography of pancreas in patients with beta-thalassemia which can indicate that the patient is developing diabetes. In this study, the aim was to investigate the findings of pancreatic sonography in patients, with beta-thalassemia major. Methods: This cross-sectional study included information extracted from the medical record and entered into an information form. A cohort of 190 people were split into case and control groups, retrospectively. The required data were extracted from patient’s documents. Data were analyzed with SPSS software, version 22; with the chi-square statistical tests. A P value of < .05 was considered statistically significant. Results: This study consisted of 33 males and 61 females in the case group and 32 males and 64 females in the control group. The most common echogenicity observed during pancreatic sonography, in the control group, was an isoechoic feature (72.3%), followed by hyper and hypoechogenicity (18.1% and 9.6%, respectively). The prevalence of an isoechoic texture in the patient case group was 58.3%, while hyper and hypoechogenicity were seen in 26% and 2.1%, respectively ( P = .070). There was a poor negative correlation between diabetes and pancreatic echogenicity ( P = .002 and r = −.226). Also, there was a poor positive correlation between fasting serum glucose levels and pancreatic echogenicity ( P = .034, n = 96 and r = .217). Conclusion: This study demonstrates, in this patient group, that there was a poor relationship between pancreatic head size with serum ferritin levels and pancreatic echogenicity with diabetes. These results may suggest using sonography to diagnose early stages of diabetes in patients with thalassemia.

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